General Information About Gastrointestinal Carcinoid Tumors

A gastrointestinal carcinoid tumor is cancer that forms in the lining of the gastrointestinal tract.

The gastrointestinal (GI) tract is part of the body's digestive system . It helps to digest food, takes nutrients (vitamins , minerals , carbohydrates , fats, proteins , and water) from food to be used by the body and helps pass waste material out of the body. The GI tract is made up of these and other organs:

• Stomach.
• Small intestine (duodenum, jejunum, and ileum).
• Colon.
• Rectum.

Gastrointestinal carcinoid tumors form from a certain type of neuroendocrine cell (a type of cell that is like a nerve cell and a hormone -making cell). These cells are scattered throughout the chest and abdomen but most are found in the GI tract. Neuroendocrine cells make hormones that help control digestive juices and the muscles used in moving food through the stomach and intestines. A GI carcinoid tumor may also make hormones and release them into the body.

GI carcinoid tumors are rare and most grow very slowly. Most of them occur in the appendix , small intestine, and rectum. Sometimes more than one tumor will form.

See the following PDQ summaries for more information related to GI and other types of carcinoid tumors:

• Non-Small Cell Lung Cancer Treatment.
• Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment.
• Rectal Cancer Treatment.
• Small Intestine Cancer Treatment.
• Unusual Cancers of Childhood

Health history can affect the risk of gastrointestinal carcinoid tumors.

Anything that increases a person's chance of developing a disease is called a risk factor . Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your doctor if you think you may be at risk.

Risk factors for GI carcinoid tumors include the following:

• Having afamily history ofmultiple endocrine neoplasia type 1 (MEN1) syndrome orneurofibromatosis type 1 (NF1) syndrome.
• Having certainconditions that affect the stomach's ability to make stomachacid , such as atrophicgastritis ,pernicious anemia , orZollinger-Ellison syndrome.

Some gastrointestinal carcinoid tumors have no symptoms in the early stages.

The growth of the tumor and/or the hormones the tumor makes may cause symptoms . Some tumors, especially tumors of the stomach or appendix, may not cause symptoms. Carcinoid tumors are often found during tests or treatments for other conditions.

Carcinoid tumors in the small intestine (duodenum, jejunum, and ileum), colon, and rectum sometimes cause symptoms as they grow or because of the hormones they make. Other conditions may cause the same symptoms. Talk to your doctor if any of the following symptoms occur.

Carcinoid syndrome may occur if the tumor spreads to the liver or other parts of the body.

The hormones made by gastrointestinal carcinoid tumors are usually destroyed by liver enzymes in the blood. If the tumor has spread to the liver and the liver enzymes cannot destroy the extra hormones made by the tumor, high amounts of these hormones may remain in the body and cause carcinoid syndrome. This can also happen if tumor cells enter the blood. Symptoms of carcinoid syndrome include the following:

• Redness or a feeling of warmth in the face and neck.
• Abdominal pain.
• Feeling bloated.
• Diarrhea.
• Wheezing or other trouble breathing.
• Fast heartbeat.

These symptoms and others may be caused by gastrointestinal carcinoid tumors or by other conditions. Talk to your doctor if any of these symptoms occur.

Imaging studies and tests that examine the blood and urine are used to detect (find) and diagnose gastrointestinal carcinoid tumors.

The following tests and procedures may be used:

• Physical exam andhistory: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
• Complete blood count: A procedure in which a sample of blood is drawn and checked for the following:
  • The number ofred blood cells ,white blood cells , andplatelets.
  • The amount ofhemoglobin (theprotein that carriesoxygen) in the red blood cells.
  • The portion of the sample made up of red blood cells.
• Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances, such as hormones, released into the blood by organs andtissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that produces it. The blood sample is checked to see if it contains a hormone produced by carcinoid tumors. This test is used to helpdiagnosecarcinoid syndrome.
• Tumor marker test: A procedure in which a sample of blood,urine , or tissue is checked to measure the amounts of certain substances, such aschromogranin A , made by organs, tissues, or tumor cells in the body. Chromogranin A is a tumor marker. It has been linked toneuroendocrine tumorswhen found in increased levels in the body.
• Twenty-four-hour urine test: A test in which a urine sample is checked to measure the amounts of certain substances, such as hormones. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that produces it. The urine sample is checked to see if it contains a hormone produced by carcinoid tumors. This test is used to help diagnose carcinoid syndrome.
• Somatostatin receptor scintigraphy (SRS): A type ofradionuclide scan used to find carcinoid tumors. In SRS,radioactive octreotide , adrug similar to somatostatin, isinjected into avein and travels through the bloodstream. The radioactive octreotide attaches to carcinoid tumor cells that have somatostatinreceptors . Aradiation -measuringdevicedetects the radioactive material, showing where the carcinoid tumor cells are in the body. This procedure is also called an octreotide scan.
• MIBGscan: A procedure used to find neuroendocrine tumors, such as carcinoid tumors. A very small amount of radioactive material called MIBG (metaiodobenzylguanidine) is injected into a vein and travels through the bloodstream. Carcinoid tumors take up the radioactive material and are detected by a device that measures radiation.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to anx-raymachine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
• MRI(magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging
• PET scan (positron emission tomography scan): A procedure to findmalignant tumor cells in the body. A small amount ofradionuclide glucose (sugar) is injected into a vein. The PETscannerrotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells.
• Endoscopic ultrasound (EUS): A procedure in which anendoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and alens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound ) off internal tissues or organs, such as the stomach, small intestine, colon, or rectum, and make echoes. The echoes form a picture of body tissues called asonogram. This procedure is also called endosonography.
• Upper endoscopy : A procedure to look at organs and tissues inside the body to check forabnormal areas. An endoscope is inserted through the mouth and passed through theesophagus into the stomach. Sometimes the endoscope also is passed from the stomach into the small intestine. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue orlymph node samples, which are checked under amicroscopefor signs of disease.
• Colonoscopy : A procedure to look inside the rectum and colon forpolyps , abnormal areas, or cancer. Acolonoscopeis inserted through the rectum into the colon. A colonoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove polyps or tissue samples, which are checked under a microscope for signs of cancer.
• Capsule endoscopy: A procedure used to see all of the small intestine. The patient swallows a capsule that contains a tiny camera. As the capsule moves through the gastrointestinal tract, the camera takes pictures and sends them to a receiver worn on the outside of the body.
• Biopsy: The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. Tissue samples may be taken during endoscopy and colonoscopy.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

• Where the tumor is in the gastrointestinal tract.
• The size of the tumor.
• Whether the cancer has spread from the stomach and intestines to other parts of the body, such as the liver or lymph nodes.
• Whether the patient has carcinoid syndrome or has carcinoid heart syndrome.
• Whether the cancer can be completely removed bysurgery.
• Whether the cancer is newly diagnosed or hasrecurred.

Stages of Gastrointestinal Carcinoid Tumors

After a gastrointestinal carcinoid tumor has been diagnosed, tests are done to find out if cancer cells have spread within the stomach and intestines or to other parts of the body.

Staging is the process used to find out how far the cancer has spread. The information gathered from the staging process determines the stage of the disease. The results of tests and procedures used to diagnose gastrointestinal carcinoid tumors may also be used for staging. See the General Information section for a description of these tests and procedures. Other tests that may be used for staging include the following:

• Angiogram : A procedure to look atblood vessels and the flow ofblood . Acontrast dye isinjected into a blood vessel near thetumor . As the contrast dye moves through the blood vessel,x-rays are taken to check the blood vessels that go to thetumor.
• Bone scan : A procedure to check if there are rapidly dividingcells , such ascancer cells, in the bone. A very small amount ofradioactive material is injected into avein and travels through the bloodstream. Theradioactive material collects in the bones and is detected by ascanner.
• X-ray of theabdomen : An x-ray of theorgans andtissues inside theabdomen. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

• Throughtissue . Cancerinvadesthe surrounding normal tissue.
• Through thelymph system . Cancer invades the lymph system and travels through thelymph vesselsto other places in the body.
• Through theblood . Cancer invades theveins andcapillariesand travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis . The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.

The plan for cancer treatment depends on where the carcinoid tumor is found and whether it can be removed by surgery.

For many cancers it is important to know the stage of the cancer in order to plan treatment. However, the treatment of gastrointestinal carcinoid tumors is not based on the stage of the cancer. Treatment depends mainly on whether the tumor can be removed by surgery and if the tumor has spread.

Treatment is based on whether the tumor:

• Can be completely removed by surgery.
• Has spread to other parts of the body.
• Has come back after treatment. The tumor may come back in thestomach orintestinesor in other parts of the body.
• Has not gotten better with treatment.

Treatment Option Overview

There are different types of treatment for patients with gastrointestinal carcinoid tumors.

Different types of treatment are available for patients with gastrointestinal (GI) carcinoid tumors . Some treatments are standard (the currently used treatment), and some are being tested in clinical trials . A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Four types of standard treatment are used:

Surgery

Treatment of gastrointestinal carcinoid tumors usually includes surgery. One of the following surgical procedures may be used:

• Endoscopic resection : Surgery to remove a smalltumor that is on the inside lining of the GI tract. Anendoscope is inserted through the mouth and passed through theesophagus to thestomach and sometimes, theduodenum . An endoscope is a thin, tube-like instrument with a light, alens for viewing, and a tool for removing tumortissue.
• Local excision: Surgery to remove the tumor and a small amount of normal tissue around it.
• Resection : Surgery to remove part or all of theorgan that contains cancer. Nearbylymph nodesmay also be removed
• Cryosurgery : A treatment that uses an instrument to freeze and destroycarcinoid tumor tissue. This type of treatment is also called cryotherapy. The doctor may useultrasoundto guide the instrument.
• Radiofrequency ablation : The use of a special probe with tiny electrodes that release high-energy radio waves (similar to microwaves) that kill cancercells . The probe may be inserted through the skin or through anincision (cut) in theabdomen.
• Liver transplant : Surgery to remove the whole liver and replace it with a healthydonatedliver.
• Hepatic artery embolization : A procedure to embolize (block) the hepatic artery, which is the mainblood vessel that bringsbloodinto the liver. Blocking the flow of blood to the liver helps kill cancer cells growing there.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds , wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

Radiopharmaceutical therapy is a type of radiation therapy. Radiation is given to the tumor using a drug that has a radioactive substance, such as iodine I 131, attached to it. The radioactive substance kills the tumor cells.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy ). When chemotherapy is placed directly into the cerebrospinal fluid , an organ , or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy).

Chemoembolization of the hepatic artery is a type of regional chemotherapy that may be used to treat a gastrointestinal carcinoid tumor that has spread to the liver. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with a substance that embolizes (blocks) the artery, and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein , which carries blood from the stomach and intestine.

The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Hormone therapy

Hormone therapy with a somatostatin analogue is a treatment that stops extra hormones from being made. GI carcinoid tumors are treated with octreotide or lanreotide which are injected under the skin or into the muscle. Octreotide and lanreotide may also have a small effect on stopping tumor growth.

Treatment for carcinoid syndrome may also be needed.

Treatment of carcinoid syndrome may include the following:

• Hormone therapy with a somatostatin analogue stops extra hormones from being made. Carcinoid syndrome is treated with octreotide or lanreotide to lessen flushing anddiarrhea. Octreotide and lanreotide may also help slow tumor growth.
• Interferon therapy stimulates the body'simmune systemto work better and lessens flushing and diarrhea. Interferon may also help slow tumor growth.
• Takingmedicinefor diarrhea.
• Taking medicine for skin rashes.
• Taking medicine to breathe easier.
• Taking medicine before havinganesthesiafor a medical procedure.

Other ways to help treat carcinoid syndrome include avoiding things that cause flushing or difficulty breathing such as alcohol, nuts, certain cheeses and foods with capsaicin , such as chili peppers. Avoiding stressful situations and certain types of physical activity can also help treat carcinoid syndrome.

For some patients with carcinoid heart syndrome, a heart valve replacement may be done.

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Several types of targeted therapy are being studied in the treatment of GI carcinoid tumors.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment Options for Gastrointestinal Carcinoid Tumors

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Carcinoid Tumors in the Stomach

Treatment of gastrointestinal (GI) carcinoid tumors in the stomach may include the following:

• Endoscopic surgery (resection ) for smalltumors.
• Surgery (resection) to remove part or all of the stomach. Nearbylymph nodesfor larger tumors, tumors that grow deep into the stomach wall, or tumors that are growing and spreading quickly may also be removed.

For patients with GI carcinoid tumors in the stomach and MEN1 syndrome, treatment may also include:

• Surgery to remove tumors in theduodenum (first part of thesmall intestine, that connects to the stomach).
• Hormone therapy.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized gastrointestinal carcinoid tumor and regional gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Carcinoid Tumors in the Small Intestine

It is not clear what the best treatment is for GI carcinoid tumors in the duodenum (first part of the small intestine , that connects to the stomach). Treatment may include the following:

• Endoscopic surgery (resection ) for smalltumors.
• Surgery (local excision) to remove the tumor, for slightly larger tumors.
• Surgery resection to remove the tumor and nearbylymph nodes.

Treatment of GI carcinoid tumors in the jejunum (middle part of the small intestine) and ileum (last part of the small intestine, that connects to the colon) may include the following:

• Surgery (resection) to remove the tumor and themembrane that connects theintestines to the back of theabdominal wall. Nearbylymph nodesare also removed.
• A second surgery to remove the membrane that connects the intestines to the back of the abdominal wall, if any tumor remains or the tumor continues to grow.
• Hormone therapy.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized gastrointestinal carcinoid tumor and regional gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Carcinoid Tumors in the Appendix

Treatment of GI carcinoid tumors in the appendix may include the following:

• Surgery (resection) to remove the appendix.
• Surgery (resection) to remove the right side of thecolon including the appendix. Nearbylymph nodesare also removed.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized gastrointestinal carcinoid tumor and regional gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Carcinoid Tumors in the Colon

Treatment of GI carcinoid tumors in the colon may include the following:

• Surgery (resection ) to remove part of the colon and nearby lymph nodes , in order to remove as much of thecanceras possible.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized gastrointestinal carcinoid tumor and regional gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Carcinoid Tumors in the Rectum

Treatment of GI carcinoid tumors in the rectum may include the following:

• Endoscopic surgery (resection ) for smalltumors.
• Surgery (local excision ) to remove slightly larger tumors that have not spread into the muscle layer of therectalwall.
• Surgery (resection) to remove the top part of the rectum and the left side of thecolon , for slightly larger tumors that have may spread to the muscle layer of the rectal wall or nearbytissues. Nearbylymph nodesare also removed.
• Surgery (local excision) to remove as much tumor as possible when the tumor has spread todistant parts of the body. This ispalliative therapy to relievesymptoms and improvequality of life.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized gastrointestinal carcinoid tumor and regional gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Metastatic Gastrointestinal Carcinoid Tumors

Distant metastases

Treatment of distant metastases of gastrointestinal carcinoid tumors is usually palliative therapy to relieve symptoms and improve quality of life. Treatment may include the following:

• Surgery (resection ) to remove as much of thetumoras possible. .
• Hormone therapy.
• Radiopharmaceutical therapy.
• Radiation therapy forcancer that has spread to the bone, brain, orspinal cord.
• Aclinical trialof a new treatment.

Liver metastases

Treatment of cancer that has spread to the liver may include the following:

• Surgery resection to remove the tumor from the liver.
• Hepatic artery embolization.
• Cryosurgery.
• Radiofrequency ablation.
• Livertransplant.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with metastatic gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Recurrent Gastrointestinal Carcinoid Tumors

Treatment of recurrent gastrointestinal carcinoid tumors may include the following:

• Surgery to remove part or all of thetumor.
• Aclinical trialof a new treatment.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent gastrointestinal carcinoid tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

To Learn More About Gastrointestinal Carcinoid Tumors

For more information from the National Cancer Institute about gastrointestinal carcinoid tumors, see the following:

• Gastrointestinal Carcinoid Tumor Home Page
• Cryosurgery in Cancer Treatment: Questions and Answers
• Understanding Cancer Series: Targeted Therapies(Advances in Targeted Therapies)
• Targeted Cancer Therapies

For general cancer information and other resources from the National Cancer Institute, see the following:

• What You Need to Know About™ Cancer
• Understanding Cancer Series: Cancer
• Cancer Staging
• Chemotherapy and You: Support for People With Cancer
• Radiation Therapy and You: Support for People With Cancer
• Coping with Cancer: Supportive and Palliative Care
• Questions to Ask Your Doctor About Cancer
• Cancer Library
• Information For Survivors/Caregivers/Advocates

Changes to This Summary (02 / 03 / 2012)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Changes were made to this summary to match those made to the health professional version.

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PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

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The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

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Last Revised: 2012-02-03

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